CCDC103 mutations cause primary ciliary dyskinesia by disrupting assembly of ciliary dynein arms

Cilia are essential for fertilization, respiratory clearance, cerebrospinal fluid circulation and establishing laterality. Cilia motility defects cause primary ciliary dyskinesia (PCD, MIM244400), a disorder affecting 1:15,000-30,000 births. Cilia motility requires the assembly of multisubunit dynein arms that drive ciliary bending. Despite progress in understanding the genetic basis of PCD, mutations remain to be identified for several PCD-linked loci. Here we show that the zebrafish cilia paralysis mutant schmalhans (smh(tn222)) encodes the coiled-coil domain containing 103 protein (Ccdc103), a foxj1a-regulated gene product. Screening 146 unrelated PCD families identified individuals in six families with reduced outer dynein arms who carried mutations in CCDC103. Dynein arm assembly in smh mutant zebrafish was rescued by wild-type but not mutant human CCDC103. Chlamydomonas Ccdc103/Pr46b functions as a tightly bound, axoneme-associated protein. These results identify Ccdc103 as a dynein arm attachment factor that causes primary ciliary dyskinesia when mutated.     

The anatomy and taxonomy of Vanclevea (Asteraceae)

Wood, leaf, and floral anatomy of Vanclevea stylosa is compared with that of several possibly related species in the genera Acamptopappus, Eastwoodia, Grindelia, Haplopappus, and Petradoria. Although V. stylosa was originally described as a Grindelia, it is clearly distinct from that genus. Of the taxa studied, it is most closely allied to Haplopappus salincinus and H. scopulorum. The taxonomy, morphology, and distribution of the monotypic Vanclevea are detailed, and known exsiccatae are listed.     

A filament of dark matter between two clusters of galaxies

It is a firm prediction of the concordance cold-dark-matter cosmological model that galaxy clusters occur at the intersection of large-scale structure filaments. The thread-like structure of this 'cosmic web' has been traced by galaxy redshift surveys for decades. More recently, the warm–hot intergalactic medium (a sparse plasma with temperatures of 10(5) kelvin to 10(7) kelvin) residing in low-redshift filaments has been observed in emission and absorption. However, a reliable direct detection of the underlying dark-matter skeleton, which should contain more than half of all matter, has remained elusive, because earlier candidates for such detections were either falsified or suffered from low signal-to-noise ratios and unphysical misalignments of dark and luminous matter. Here we report the detection of a dark-matter filament connecting the two main components of the Abell 222/223 supercluster system from its weak gravitational lensing signal, both in a non-parametric mass reconstruction and in parametric model fits. This filament is coincident with an overdensity of galaxies and diffuse, soft-X-ray emission, and contributes a mass comparable to that of an additional galaxy cluster to the total mass of the supercluster. By combining this result with X-ray observations, we can place an upper limit of 0.09 on the hot gas fraction (the mass of X-ray-emitting gas divided by the total mass) in the filament.     

Das statistische Problem der Gestalt fadenförmiger Moleküle

Ohne Zusammenfassung     

Purification of adenoviruses by the fluorocarbon procedure

Résumé La purification préalable, par le fluorocarbone, d'adénovirus (types 3, 4, 5 et 7) servant à l'immunisation de lapins, prévient la formation dans le sérum de ces derniers d'anticorps fixateurs de complément, réagissant de manière non spécifique avec les constituants des cultures de cellules dans lesquelles les virus ont été propagés. Cette méthode ne permet cependant pas d'obtenir des anticorps qui réagissent de manière spécifique de type avec les adénovirus, dans la réaction de déviation du complément.      

Quantification of the disorder in network-modified silicate glasses.

Local order in silicate glasses has been observed by many experimental techniques to be similar to that in crystalline materials. Details of the intermediate-range order are more elusive, but essential for understanding the lack of long-range symmetry in glasses and the effect of composition on glass structure. Two-dimensional 17O dynamic-angle-spinning nuclear magnetic resonance experiments reveal intermediate-range order in the distribution of inter-tetrahedral (Si-O-Si) bond angles and a high degree of order in the disposition of oxygen atoms around the network-modifying cations.